Edson J R, Vogt J M, Hasegawa D K
Blood. 1984 Oct;64(4):807-16.
Prothrombin deficiency has been known to occur in association with lupus inhibitors for over 25 years. We studied 21 patients with lupus inhibitors and found that four of five with prothrombin deficiency and ten of 16 with quantitatively normal prothrombin had abnormal prothrombin crossed-immunoelectrophoresis (CIEP) characterized by material moving slower in the first dimension of electrophoresis than normal prothrombin. In two patients with prothrombin deficiency, all prothrombin measured by quantitative assay and all slow-moving material on CIEP were removed by treatment with Staphylococcal protein A (SPA). These patients had free antibody, which bound to normal plasma prothrombin, forming larger amounts of slow-moving material on CIEP. A third patient with prothrombin deficiency had only partial removal of prothrombin after SPA treatment. Two patients with quantitatively normal prothrombin had all slow-moving material on CIEP and about one fourth of the prothrombin by quantitative assay removed by SPA treatment. There was no correlation among the strength of the inhibitor, the presence of a "cofactor effect," and the prothrombin abnormality. These data suggest that heterogeneous antiprothrombin antibodies, with or without prothrombin deficiency, are present in the majority of patients with lupus inhibitors.
已知凝血酶原缺乏与狼疮抑制剂相关，这种情况已存在超过25年。我们研究了21例狼疮抑制剂患者，发现5例凝血酶原缺乏患者中有4例，以及16例凝血酶原定量正常患者中有10例，其凝血酶原交叉免疫电泳（CIEP）异常，表现为在电泳第一维中移动速度比正常凝血酶慢的物质。在2例凝血酶原缺乏患者中，通过用葡萄球菌蛋白A（SPA）治疗，定量测定的所有凝血酶原以及CIEP上所有移动缓慢的物质都被清除。这些患者有游离抗体，其与正常血浆凝血酶原结合，在CIEP上形成大量移动缓慢的物质。第三例凝血酶原缺乏患者在SPA治疗后凝血酶原仅部分被清除。2例凝血酶原定量正常的患者，其CIEP上所有移动缓慢的物质以及定量测定约四分之一的凝血酶原通过SPA治疗被清除。抑制剂强度、“辅因子效应”的存在与凝血酶原异常之间没有相关性。这些数据表明，大多数狼疮抑制剂患者存在异质性抗凝血酶原抗体，无论有无凝血酶原缺乏。
